Pediatric Renal Flashcards

1
Q

Alport syndrome occurs secondary to an inherited defect in type […] collagen.

A

Alport syndrome occurs secondary to an inherited defect in type IV collagen.

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2
Q

Chronic constipation in toddlers may be complicated by recurrent UTIs, secondary to urinary […].

A

Chronic constipation in toddlers may be complicated by recurrent UTIs, secondary to urinary stasis.

impacted stool can cause rectal distention, which compresses the bladder and prevents complete voiding

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3
Q

Classic features of new-onset type 1 diabetes mellitus include […] and […].

A

Classic features of new-onset type 1 diabetes mellitus include polydipsia and polyuria.

typically manifests in children age 4 - 6 years old or at early puberty (bimodal onset)

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4
Q

Henoch-Schonlein purpura is a leukocytoclastic vasculitis that occurs secondary to […] immune complex deposition.

A

Henoch-Schonlein purpura is a leukocytoclastic vasculitis that occurs secondary to IgA immune complex deposition.

often seen as deposition of IgA in the mesangium on renal biopsy

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5
Q

In Potter sequence, oligohydramnios leads to pulmonary […].

A

In Potter sequence, oligohydramnios leads to pulmonary hypoplasia.

most common cause of death in infants with Potter sequence; due to the lack of normal alveolar distention by aspirated amniotic fluid

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6
Q

Infants in diapers with suspected UTI should undergo […] to obtain a sterile specimen for urinalysis and urine culture.

A

Infants in diapers with suspected UTI should undergo straight catheterization to obtain a sterile specimen for urinalysis and urine culture.

clean-catch specimens are unreliable in diapered patients due to high likelihood of contamination with stool or skin flora

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7
Q

Renal tubular acidosis in infants commonly presents with failure to thrive in the setting of […]. (acid-base abnormality)

A

Renal tubular acidosis in infants commonly presents with failure to thrive in the setting of normal anion gap metabolic acidosis. (acid-base abnormality)

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8
Q

Renal tubular acidosis is caused by a defect in either […] (type 1) or […] (type 2) in the kidney.

A

Renal tubular acidosis is caused by a defect in either hydrogen excretion (type 1) or bicarbonate resorption (type 2) in the kidney.

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9
Q

The primary abnormality in Potter sequence is […]-hydamnios.

A

The primary abnormality in Potter sequence is oligo-hydamnios.

low amniotic fluid subsequently results in pulmonary hypoplasia, flat facies, and limb deformities

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10
Q

Urinary tract infection typically arises by bacteria […] into the bladder from the vaginal introitus.

A

Urinary tract infection typically arises by bacteria ascending into the bladder from the vaginal introitus.

often precipitated by sexual intercourse (“honeymoon cystitis”)

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11
Q

What acid-base disturbance is associated with pyloric stenosis?

A

Hypokalemic, hypochloremic metabolic alkalosis

secondary to vomiting of gastric acid and subsequent volume contraction; should be corrected prior to pyloromyotomy to reduce risk of post-operative apnea

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12
Q

What childhood malignancy typically manifests as an asymptomatic abdominal mass that does not cross the midline?

A

Wilms tumor (nephroblastoma)

may also present with hematuria; versus neuroblastoma, which is typically symptomatic and crosses the midline

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13
Q

What inherited renal pathology is characterized by thinning and splitting of the glomerular basement membrane?

A

Alport syndrome

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14
Q

What is the first-line pharmacotherapy for nocturnal enuresis?

A

Desmopressin

typically reserved for patients age > 5 whose symptoms are not controlled with lifestyle changes or enuresis alarms

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15
Q

What is the fluid of choice for initial resuscitation of severe hypovolemic hypernatremia in an infant?

A

Isotonic solutions (e.g. normal saline, lactated Ringer’s)

using hypotonic solutions initially may lower the sodium too rapidly

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16
Q

What is the likely diagnosis in a child that presents with abdominal pain, arthralgias, and palpable purpura on the lower extremities?

A

Henoch-Schonlein purpura

often renal involvement occurs, which can result in hematuria

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17
Q

What is the likely diagnosis in a young girl that presents with recurrent UTIs since birth with bilateral focal parenchymal scarring and blunted calyces on imaging?

A

Vesicoureteral reflux

imaging findings are consistent with recurrent for chronic pyelonephritis

18
Q

What is the likely diagnosis in a young male that presents with recurrent hematuria and bilateral sensorineural deafness?

A

Alport syndrome

classically presents as a triad of visual, renal, and hearing problems; “can’t see, can’t pee, can’t hear a bee”

19
Q

What is the likely diagnosis in an adolescent that presents with features of nephrotic syndrome and hepatitis B infection?

A

Membranous nephropathy

MPGN is also associated with hepatitis B, however it is much less common than membranous nephropathy

20
Q

What is the likely diagnosis in an African-American patient that presents with painless gross hematuria with numerous intact RBCs on microscopic examination?

A

Renal papillary necrosis (secondary to sickle cell trait)

relatively low partial pressure of O2 in the vasa rectae of the medulla predispose RBCs to sickling, which increases risk for papillary necrosis

21
Q

What is the most common cause of nephrotic syndrome in preadolescent children (< 10 years old)?

A

Minimal change disease

22
Q

What is the most common cause of proteinuria in children?

A

Transient proteinuria

typically benign; may be precipitated by fever, exercise, seizure, stress, and volume depletion

23
Q

What is the most common cause of urinary tract obstruction in newborn boys?

A

Posterior urethral valves

classic findings on prenatal US include bladder distension and bilateral hydronephrosis; this condition does not occur in girls

24
Q

What is the most common GI complication associated with Henoch-Schonlein purpura (besides abdominal pain)?

A

Ileoileal intussusception

secondary to intestinal edema and bleeding

25
Q

What is the most common renal malignancy of early childhood (ages 2-5)?

A

Wilms tumor (nephroblastoma)

26
Q

What is the most common renal manifestation of Henoch-Scholein purpura?

A

Hematuria

27
Q

What is the most common site of metastatic spread of a Wilms tumor?

A

Lungs

thus, children with suspected Wilms tumor should have chest imaging to evaluate for metastases

28
Q

What is the next step in management for a 12-month-old girl with a confirmed E. coli UTI that is resolving after initiation of antibiotics?

A

Renal and bladder ultrasound

29
Q

What is the next step in management for a child that is found to have isolated proteinuria on urine dipstick?

A

Repeat dipstick testing on two subsequent occasions

if subsequent tests are negative, the diagnosis is transient proteinuria; if subsequent tests reveal continued proteinuria, further workup is warranted

30
Q

What is the next step in management for a child with suspected minimal change disease?

A

Corticosteroids

31
Q

What is the next step in management for a patient that needs emergency fluid resuscitation and peripheral IV access cannot be obtained?

A

Attempt intraosseous access

requires less skill and practice than central line placement and is safer and faster

32
Q

What is the recommended treatment for Henoch-Schonlein purpura?

A

supportive (e.g. hydration, NSAIDs); treat with steroids if severe

33
Q

What is the test of choice for definitive diagnosis of vesicoureteral reflux?

A

Voiding cystourethrogram

typically done for newborns age < 1 month with their first UTI, children < 2 years with recurrent UTIs, or a first UTI from an organism other than E. coli

34
Q

What microorganism is the most common cause of urinary tract infection (UTI)?

A

Escherichia coli

35
Q

Which type(s) of renal tubular acidosis are associated with high serum K+?

A

Type 4 (hyperkalemic)

due to aldosterone resistance or decreased aldosterone production

36
Q

Which type(s) of renal tubular acidosis are associated with low or normal serum K+?

A

type 1 (distal) and type 2 (proximal)

37
Q

Which type(s) of renal tubular acidosis are associated with urine pH <5.5?

A

type 2 (proximal) and type 4

38
Q

Which type(s) of renal tubular acidosis are associated with urine pH > 5.5 (alkalotic)?

A

Type 1 (distal)

high urine pH increases the risk for calcium phosphate kidney stones

39
Q

WAGR syndrome is a combination of:

  1. […]
  2. Aniridia (absence of iris)
  3. Genital abnormalities
  4. mental or motor Retardation
A

WAGR syndrome is a combination of:

  1. Wilms tumor
  2. Aniridia (absence of iris)
  3. Genital abnormalities
40
Q

WAGR syndrome is a combination of:

  1. Wilms tumor
  2. […]
  3. Genital abnormalities
  4. mental or motor Retardation
A

WAGR syndrome is a combination of:

  1. Wilms tumor
  2. Aniridia (absence of iris)
  3. Genital abnormalities
  4. mental or motor Retardation
41
Q

WAGR syndrome is a combination of:

  1. Wilms tumor
  2. Aniridia (absence of iris)
  3. […]
  4. mental or motor Retardation
A

WAGR syndrome is a combination of:

  1. Wilms tumor
  2. Aniridia (absence of iris)
  3. Genital abnormalities
  4. mental or motor Retardation
42
Q

WAGR syndrome is a combination of:

  1. Wilms tumor
  2. Aniridia (absence of iris)
  3. Genital abnormalities
  4. […]
A

WAGR syndrome is a combination of:

  1. Wilms tumor
  2. Aniridia (absence of iris)
  3. Genital abnormalities
  4. mental or motor Retardation